Kidney Cancer - General Overview

Front 1

According to Campbell's chart in chapter 47 on renal tumors which of the Bosniak category cysts enhance?

Back 1

*According to Campbell's only category IV cysts enhance.

Front 2

Who is more likely to have a benign renal mass a man or a woman?

Back 2

*A woman, and in one series women with renal masses suggestive of RCC were found to have benign path in 36% of cases. (Eggener et al. 2004)

Front 3

How often is a radiographically and clinically suggestive RCC found to be benign after surgical extirpation?

Back 3

10-20% of small, solid, enhancing masses are found to be benign.

Front 4

What is a metanephric adenoma? What chromosomal abnormality can be associated with metanephric adenomas, and what other subtype of RCC is associated with the same abnormality?

Back 4

A metanephric adenoma is a pathologic diagnosis. Highly basophilic epithelial cells are seen on histology. These adenomas are rarely if ever metastatic, but because they can’t be reliably diagnosed clinically or radiographically they are surgically removed because of the concern for malignancy.

Polysomy of chromosomes 7 and 17, suggesting a clonal neoplastic disorder associated with metanephric adenoma and potentially related to papillary RCC

Front 5

What is a renal oncocytoma? From which renal cell type does the oncocytoma arise? What subtype of RCC has a histologic similarity to oncocytoma and why?

Back 5

A benign renal mass that represents 3-7% of all solid renal masses which are light brown or tan, well circumscribed, homogenous in appearance, and may contain a central scar. It arises from the distal tubule. Chromophobic RCC also arises from the distal tubule and is histologically similar, especially the eosinophilic variant of chromophobic carcinoma.

Front 6

Can oncocytoma be differentiated from RCC with radiographic studies? How should renal oncocytomas be treated?

Back 6

No. Given the uncertainty of the pre-operative diagnosis and the fact that RCC may be present in oncocytoma masses, these lesions should be surgically removed or ablated.

Front 7

What is an AML?

Back 7

Benign neoplasm consisting of varying amounts of mature adipose tissue, smooth muscle, and thick walled vessels.

Front 8

What is Wunderlich’s syndrome? What increases the risk of hemorrhage from AML? Who should be treated if they have an AML?

Back 8

Massive retroperitoneal hemorrhage caused by AML which is found in up to 10% of patients with AML.

Pregnancy increases the risk of hemorrhage from AML. Treat those with AMLs greater than or equal to 4 cm with symptoms, women of child bearing age, those who don’t have access to good emergency care.

Front 9

Immunoreactivity for what monoclonal antibody is characteristic of AML, and how is it used?

Back 9

Positive reaction to HMB-45 is characteristic for AML and can be used to differentiate AML from sarcoma

Front 10

What is considered diagnostic radiographically of AML?

Back 10

The presence of even a small amount of fat on CT (-20 HU or lower) virtually excludes RCC and diagnostic

Front 11

What is a cystic nephroma? Explain the differences in the ages at presentation and symptoms in these age groups for cystic nephroma. How are cystic nephromas managed?

Back 11

A cystic nephroma is a benign multilocular renal mass with a bimodal age distribution that has a characteristic hobnailed pattern microscopically. It typically is classified as a Bosniack III or IV renal cyst. There is a male predominance is observed in children, and a female predominance in adults. Children present with an asymptomatic abdominal mass, while adults present with symptomatic abdominal pain, hematuria, UTI, or hypertension

Treat with radical nephrectomy because of the concern for cystic RCC in adults and Wilms in kids. Pre-op radiographic studies can’t reliably r/o malignancy.

Front 12

What diagnosis must be considered in any patient with hyperreninemia and hypertension in whom renal artery stenosis has been excluded? What other symptoms can be present?

Back 12

Juxtaglomerular tumor (reninoma) are often small (< 3cm), solitary, and hypovascular, i guess potentially like pap type 1 tumors.. They secrete rennin and present with hypertension, hypokalemia, polydipsia, polyuria, myalgia, and headaches.

Front 13

What is the difference in terms of surgical management of RCC in young adults and children?

Back 13

A lymphadenectomy should be performed for improved survival in this population.

Front 14

What is von-Hippel Lindau disease? What are the major manifestations of RCC? What other symptoms can be present?

Back 14

It is the familial form of the common clear cell variant of RCC. The major manifestations of RCC include development of RCC in about 50% of patients with VHL, often with an early age of onset, often bilateral and multifocal involvement. Other symptoms include pheochromocytomas, retinal angiomas, hemangioblastomas of the brain stem, cerebellum, or spinal cord, renal and pancreatic cysts, inner ear tumors, and papillary cystadenomas of the epididymis.

So the familial syndromes can be remembered with the following mneumonic that there are 5, like the 5 little piggies. One is BAD for (BHD), looks like michael jackson from the bad videos, one is a Vampire (VHL), one is a honry pervert (HPRC), with w trenchcoat and he is like a flasher, and one is a Hippy Loser (HLRC), like the hippy guy chong from that 70's show.

Front 15

Where is the VHL tumor suppressor gene located? How does a VHL mutation lead to RCC?

Back 15

Chromosome 3p25.

The VHL protein complex targets hypoxia-inducible factor 1 (HIF-1) for degradation, keeping HIF-1 levels low under normal conditions. HIF-1 plays an important role in regulating cellular responses to hypoxia, starvation, and other stresses. The VHL mutation leads to increased intracellular HIF-1, which leads to upregulation of vascular endothelial growth factor (VEGF), the proangiogenic growth factor in RCC.

Front 16

What is Birt-Hogg-Dube syndrome?

Back 16

This is a syndrome in which patients develop cutaneous fibrofolliculomas, lung cysts, spontaneous pneumothoraces, and a variety of renal tumors primarily derived from the distal tubule, including chromophobe RCC, oncocytomas, and hybrid or transitional tumors.

Front 17

1. What is generic name for Tarceva?How does Tarceva work?
2. How does it work?

Back 17

1. Erlotinib
2. It targets EGFR (epidermal growth factor receptor)

Hint: Maybe you can remember what it does by trade name in that it TARgets the EGFR receptor.

Front 18

Name the histologic types of RCC and their respective percentage breakdowns. Which histologic type of RCC portends the worst prognosis, conventional, chromophilic, or chromophobic?

Back 18

Conventional (70-80%), chromophilic aka papillary (10-15%), chromophobic (3-5%), collecting duct (1%), unclassified (1%). Conventional has the worst prognosis.

Front 19

What cytogenetic abnormalities are associated with chromophilic/papillary RCC? Is chromophilic/papillary usually multicentric or solitary?

Back 19

Trisomy of chromosomes 7 and 17 and loss of the Y chromosome. Activating mutations of the met proto-oncogene on chromosome 7q31 is another common, pathogenic feature of chromophilic RCC. Multicentric tumors (multiple tumors arising independently) occur in 40% of cases.

Front 20

Who are more predisposed to the papillary histologic type of RCC? What is the single most distinctive and defining pathologic feature of chromophobic RCC?

Back 20

Those with ESRD or acquired renal cystic disease. Microvesicles are most defining feature.

Front 21

What population is renal medullary carcinoma prevalent in?

Back 21

African Americans with sickle cell trait, often diagnosed in the third decade of life

Front 22

What is the classic (or “too late”) triad for the presentation of RCC? How do at least half of RCCs present?

Back 22

The classic triad is flank pain, gross hematuria, and a palpable abdominal mass. This is rarely found because of early detection with increased use of CT and other radiographic studies. 50% found incidentally.

Front 23

How often are paraneoplastic syndromes found in patients with RCC?

Back 23

20% of patients with RCC have paraneoplastic syndromes with the production of parathyroid like hormone peptides leading to hypercalcemia being the most common.

Paraneoplastic syndromes with RCC are usually NICE (20), in that they go away after surgery.

Front 24

How do you treat hypercalcemia associated with RCC?

Back 24

Hydration and lasix, and if severe bisphosphonates and calcitonin

Front 25

What is Stauffer’s syndrome? What is found on liver biopsy with Stauffer’s syndrome?

Back 25

Nonmetastatic hepatic dysfunction leading to elevated LFTs present in 3-20% of cases of RCC. Hepatic function normalizes 60-70% of the time after radical nephrectomy. If you have a persistent elevation of LFTs, you are dealing with metastatic disease. On pathology: nonspecific hepatitis associated with a prominent lymphocytic infiltrate.

Front 26

How often should patients with autosomal dominant polycystic kidney disease (ADPCKD) be imaged to screen for RCC and why?

Back 26

Never. There is no increased risk for RCC in ADPCKD, and imaging is difficult related to the altered intrarenal architecture.

Front 27

What patients should be targeted for screening for RCC? When should patients with ESRD begin screening for RCC?

Back 27

Patients with ESRD, acquired renal cystic disease, TS, VHL, and familial RCC. The third year on dialysis

Front 28

How should you image a patient if you suspect an IVC thrombus?

Back 28

MRI, although multiplanar CT may be equivalent. Venacavography is reserved for patients with equivocal MRI findings or with MRI contraindications.

Front 29

What is the single most important prognostic factor for RCC?

Back 29

Pathologic stage

Front 30

Define a radical nephrectomy.

Back 30

A radical nephrectomy includes removing the kidney while it is in Gerota’s fascia, performing a regional lymphadenectomy, and removing the ipsilateral adrenal gland. We do not perform a regional lymphadenectomy and we only remove the ipsilateral adrenal gland if it is involved on pre-operative imaging.

Front 31

When can laparoscopic radical nephrectomy be considered?

Back 31

Low- to moderate-volume (8-10cm), localized RCCs with no local invasion, renal vein involvement, or lymphadenopathy

Front 32

What is the recommended post-operative surveillance after nephrectomy for localized RCC?

Back 32

Yearly H&P with blood tests for T1N0M0. Yearly H&P, blood test, cxray for T2N0M0 and every 2 years CT. For T3N0M0, H&P/blood tests and chest x-ray every 6 months for 3 years, then yearly. At 1 year should get abdominal CT, then every 2 years (radical). Every 6 months for 3 years, then ever 2 years (partial).

Front 33

You are seeing a patient in follow-up that is status post partial nephrectomy on a solitary kidney. This patient had a urinalysis today that showed 2+ proteinuria with a normal creatinine. What evaluation or follow-up should be done for this patient?

Back 33

*This patient may have hyperfiltration injury. This may occur after partial nephrectomy because of the increased filtration of the remainder of the kidney. Patients may have proteinuria, FSGS, and renal failure. Treated with dietary restriction of protein and ACE inhibitors.

According to an SASP question from 2010 you would want a 24 hour urinary protein on this patient to decide on whether or not to put him on ace inhibitor or protein restriciton. The creatinine is a poor measurement for kidney function or damage as it may stay normal initially even in a solitary kidney.

Chronic renal insufficiency in humans usually progresses to
end-stage renal failure. A decade ago, on the basis of studies in animals, Brenner and colleagues proposed that maladaptive gbmerular hemodynamic changes exert a major influence on the factors that initiate and perpetuate disease progression [1, 2]. These hemodynamic changes lead to glomerular hyperfiltration, an adaptation seen in response to a reduction in functional nephron number whether induced genetically, surgically, or by acquired renal disease. The elevated single nephron glomerular filtration rate (SNGFR) common to these pathophysiologic conditions is usually caused by increases in the glomerular capillary plasma flow rate (QA) and mean glomerular capillary hydraulic pressure (P), which in turn are due to adaptive reductions in pregbomerular and postglomerular arteriolar resistances. Progressive glomerular sclerosis and proteinuria eventually occur in most experimental models of renal disease characterized by glomerular hyperfiltration and hypertension.

source - The hyperfiltration theory: A paradigm shift in nephrology. BARRY M. BRENNER, ELIZABETH V. LAWLER, and HARALD S. MACKENZIE Renal Divisin, Department of Medicine, Brigham and Women's Hospital, Hanard Medical School, Boston, Massachusetts, USA

Front 34

Who is an ideal candidate for thermal ablative therapies for RCC? What are some disadvantages of thermal ablative therapies?

Back 34

Those who are not good operative candidates from advanced age or significant comorbidities, multiple renal masses for which multiple partials would be cumbersome or impossible, those who have local recurrence after nephron sparing surgery. Disadvantages: Local recurrence rate may be somewhat higher, you don’t get tissue for pathologic staging, and long term efficacy is not established.

Front 35

When can observation be proposed as a treatment option?

Back 35

Patients with small (<3cm), solid, enhancing, well-marginated, homogeneous renal lesions, who are elderly or poor surgical risks can be managed with serial renal imaging at 6-12 month intervals

Front 36

How often does IVC involvement with RCC occur? What is the cure rate?

Back 36

IVC involvement occurs in 4-10% of patients and 45-70% of patients with RCC and IVC thrombus can be cured with radical nephrectomy and IVC thrombectomy.

Remember the numbers with the saying that IVC involvement is a LOW (5) number but don't roll the DICE (10), because if you LOSE (50) you can KISS (70) yourself goodbye.

Front 37

What clinical symptoms would make you worry about an IVC thrombus? Is it possible to tell if an IVC thrombus is invading the wall radiographically?

Back 37

RCC patients with lower extremity edema, an isolated right-sided or non-reducible varicocele, dilated superficial abdominal veins, proteinuria, or a PE. No, unfortunately this bad prognostic indicator is seen intraoperatively.

Front 38

What’s the incidence of local recurrence after radical nephrectomy, and how often is this recurrence isolated?

Back 38

2-4% of cases. Only about 40% of local recurrences are isolated, therefore a thorough metastatic workup is necessary.

Front 39

What is the role for radiation with RCC?

Back 39

At present, the main role of radiation therapy for patients with metastatic RCC is for the palliation of symptomatic osseous metastases (Halperin and Harisiadis, 1983; Cutuli et al, 1990).
Radiation therapy has also been combined with surgery for the treatment of vertebral body metastases with spinal cord compression, allowing substantially reduced blood loss (Sundaresan et al, 1986).

Standard radiation therapy may also be employed for palliation of brain metastases (Halperin and Harisiadis, 1983; Maor et al, 1988; Cutuli et al, 1990).

Stereotactic radiosurgery has shown promise for the treatment of select patients with brain metastases, thereby avoiding the risks associated with craniotomy (Coffey et al, 1991).

Front 40

1.)What are two cytokines that are used as therapy for metastatic RCC, and what are their response rates?

2.)How does Bevacizumab (Avastin) work?

3.)How does Sorafenib (Nexavar) work?

Back 40

1.)Interferon alpha and IL-2. Interferon alpha monotherapy provides a response of 12%, with an improvement in median survival of 3.8 months. IL-2 has an overall response rate of 15%. Together they have a 19% response rate, but no improvement in overall survival.

2.) It is an IgG1 monoclonal antibody that binds all VEGF isoforms, habiting signaling through the VEGF pathway by sequestering the ligand. So mneumonic is that it is a monoclonal antibody which is the last three letters. the first letter is B - binds then e - every then V - VEGF receptors.

3.)It is an oral tyrosine kinase inhibitor with an excellent toxicity profile. You can at least remember that it is oral since the letters ora are together in the name.

Front 41

S.W. is a 65 yo male that presented w/ weight loss, two pulmonary nodules of unknown significance as well as a 9cm renal lesion with symptomatic discomfort. For a number of social reasons it took the patient about a month and a half to get to the OR. In the OR he was found to have a 28cm mass that was very very firm, almost hard as a rock. We began the case pure lap and converted to hand-assist again noting the firmness and heaviness of the mass. What would you guess was this patient's pathology based on clinical history? How common are these types of renal masses?

Back 41

*Patient had renal leiomyosarcoma which is very rare, only 1-2% of renal tumors are sarcomas, most commonly leiomyosarcomas, with a peak presentation in the fifth decade of life. If you have a patient with a rapidly expanding tumor that looks like it is arising from the capsule a sarcoma should be in your differential. The most common site of metastasis is the lung and patients usually die within months. Not a good prognosis. This patient was offered chemotherapy as a palliative option.

Front 42

Is renal lymphoma more common with non-Hodgkin’s or Hodgkin’s lymphoma?

Back 42

Non-Hodgkin’s lymphoma.

Front 43

What are the most common malignant neoplasms in the kidney?

Back 43

Mets by a wide margin, with 12% of patient dying of cancer with renal metastases

Front 44

1. How common is local recurrence after radical nephrectomy for renal cell cancer?

Back 44

1. First you need to remember that ths question is asking about local recurrence after radical nephrectomy not partial nephrectomy. Most of the series that looked at local recurrence after partial nephrectomy, initially with pT1a was 1-5% then pT1b was around 1-5%. This recurrence rate went up with positive margin, but not dramatically.
Local recurrence of RCC after radical nephrectomy is an uncommon event, occurring in approximately 2% to 4% of cases.

Front 45

What are risk factors for local recurrence after radical nephrectomy for RCC?

Back 45

Risk factors include increasing T stage and locally advanced or node-positive disease.

Front 46

What percentage of patients with local recurrence also have metastatic disease at presentation with their recurrence?

Back 46

Only about 40% of local recurrences are isolated; the majority of patients with local recurrence also have systemic disease, and a thorough metastatic evaluation should be pursued.

Front 47

Ms N.S. is 3 years out from a radical nephrectomy with T3b disease w/ invasion of the tumor thrombus into the intima of the IVC. Nodal disseciton was not performed. She presents back after surveillance imaging showed evidence of local recurrence at her IVC in the renal fossa. Her adrenal gland was removed. A thorough metastatic evaluation has been performed and there is no evidence of disease elsewhere. Is there a role for surgery in this patient and if so what is the long term cancer free rate?

Back 47

Isolated local recurrence of RCC after radical nephrectomy should be considered for surgical resection, which can provide long-term cancer-free status for about 30% to 40% of patients with this dilemma.

However, this is often a formidable task because the natural tissue barriers are no longer present and invasion of contiguous organs is not uncommon. En bloc resection of adjacent organs is often required, and the risk of morbidity can be substantial.

Front 48

Ms N.S. is 3 years out from a radical nephrectomy with T3b disease w/ invasion of the tumor thrombus into the intima of the IVC. Nodal disseciton was not performed. She presents back after surveillance imaging showed evidence of local recurrence at her IVC in the renal fossa. Her adrenal gland was removed. A thorough metastatic evaluation has been performed and there is no evidence of disease elsewhere. This patient is 81 and with several co-morbidites is not an operative candidate. Is there anything that can be done for her?

Back 48

Radiation therapy may be of value for palliation of symptomatic local recurrence in patients who are not operative candidates.

Front 49

Based on the AUA newest guidleines for the management of small renal masses what is the role for renal biopsy?

Back 49

Percutaneous renal biopsy or fine needle aspiration (FNA) has traditionally served a limited role in the evaluation of renal masses because of the relatively high diagnostic accuracy of crosssectional imaging such as CT or MRI and concern about a high false-negative rate and potential complications associated with renal mass biopsy.

Biopsy or aspiration was thus primarily reserved for patients suspected of having renal metastasis, abscess or lymphoma, or when needed to establish a pathologic diagnosis of RCC in occasional patients presenting with disseminated metastases or unresectable primary tumors. In recent years, the potential role of biopsy for localized renal tumors has been revisited,
in part driven by the recognition that 20% clinical stage T1 renal masses may represent benign disease and could be considered for less aggressive management. In addition, accuracy and safety of renal mass biopsy has improved substantially due to further refinements in CT- and
MRI-guided techniques.39-46 A review of studies since 2001 demonstrates that the false-negative rate with renal mass biopsy is now only 1%, and the incidence of symptomatic complications is relatively low, with only a very small percentage (< 2%) requiring any form of intervention.

Front 50

What is a MEST of the kidney?

Back 50

*This is a mixed epithelial stromal tumor of the kidney. The course is uniformally benign. At computed tomography (CT), it typically manifests as a multiloculated cystic renal mass with a variable proportion of solid and cystic components and containing internal septa that demonstrate heterogeneous and delayed contrast material enhancement. MEST may mimic a variety of benign and malignant renal lesions, such as adult cystic nephroma, complex renal cyst, and cystic renal cell carcinoma.

These tumors can express estrogen receptors.